Bullous Pemhpigoid (BP) ELISA Kit for Rat

This gene encodes a member of the plakin protein family of adhesion junction plaque proteins. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene, but the full-length nature of some variants has not been defined. It has been reported that some isoforms are expressed in neural and muscle tissue, anchoring neural intermediate filaments to the actin cytoskeleton, and some isoforms are expressed in epithelial tissue, anchoring keratin-containing intermediate filaments to hemidesmosomes. Consistent with the expression, mice defective for this gene show skin blistering and neurodegeneration. [provided by RefSeq, Mar 2010]

BPAG1 iso3: a protein of the plakin family. Plakins are multi-domain proteins that have been shown to interact with microtubules, actin filaments and intermediate filaments, as well as proteins found in cellular junctions. Ten alternatively spliced human isoforms have been reported. BPAG1 has neuronal and muscle isoforms that consist of actin-binding and microtubule-binding domains at either end separated by a plakin domain and many spectrin repeats. Bpag1 isoforms with different N-termini may have differing roles. The plakin domain contains an apparent nuclear localization signal.

Protein type: Motility/polarity/chemotaxis; Actin-binding; Microtubule-binding; Cytoskeletal

Chromosomal Location of Human Ortholog: 6p12.1

Cellular Component: endoplasmic reticulum membrane; intermediate filament cytoskeleton; focal adhesion; leading edge; cytoplasmic membrane-bound vesicle; integral to membrane; intermediate filament; nuclear envelope; cell cortex; cytosol; Z disc; actin cytoskeleton; microtubule cytoskeleton; hemidesmosome; axon; cytoplasm; basal plasma membrane; basement membrane; nucleus

Molecular Function: protein C-terminus binding; integrin binding; microtubule plus-end binding; protein binding; protein homodimerization activity; calcium ion binding; actin binding

Biological Process: integrin-mediated signaling pathway; extracellular matrix organization and biogenesis; hemidesmosome assembly; response to wounding; cell motility involved in cell locomotion; intermediate filament cytoskeleton organization and biogenesis; cytoskeleton organization and biogenesis; microtubule cytoskeleton organization and biogenesis; maintenance of cell polarity; cell cycle arrest; cell adhesion